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• W3206128979 abstract "Objective: To compare clinical characteristics of sporadic gastrinoma and multiple endocrine neoplasia type 1 (MEN1)-related gastrinoma. Methods: A retrospective cohort study was conducted. Patients with clinical manifestations of Zollinger-Ellison syndrome, pathological diagnosis as neuroendocrine neoplasm (NEN) and complete clinical and follow-up data were enrolled. Patients with only high gastric acid secretion but without evidence of NEN, or with other concurrent non-NEN tumors were excluded. According to the above criteria, the clinicopathological data of 52 cases of gastrinoma diagnosed from April 2003 to December 2020 in the First Affiliated Hospital, Sun Yat-sen University, were collected. Patients who met the diagnostic criteria of gastrinoma and met one of the following conditions were diagnosed as MEN1-related gastrinoma: (1) the presence of pathogenic mutations in the MEN1 gene confirmed by genetic testing; (2) NENs involving two or more endocrine glands, namely, pituitary, parathyroid, thymic, pancreatic, and adrenal NENs; (3) NEN and at least one first-degree relatives diagnosed as MEN1. The remaining gastrinomas were defined as sporadic gastrinoma. Student's t test and chi-square test were used for statistical analysis. Clinicopathological characteristics, endoscopic findings, imaging characteristics, treatment, and prognosis of sporadic and MEN1-related gastrinoma were compared. Results: Among 52 patients with gastrinoma, 33 were sporadic gastrinoma and 19 were MEN1-related gastrinoma. The common symptoms of both sporadic and MEN1-related gastrinomas were diarrhea (24/33, 72.7%; 17/19, 89.5%) and abdominal pain (19/33, 57.6%; 9/19, 47.4%). Compared with sporadic gastrinoma, MEN1-related gastrinoma needed longer time for diagnosis [(7.4±4.9) years vs. (3.9±5.2) years, t=-2.355, P=0.022), were more likely multiple tumors [47.4% (9/19) vs. 15.2% (5/33), χ(2)=6.361, P=0.012], had smaller diameter [(1.7±1.0) cm vs. (3.1±1.8) cm, t=2.942, P=0.005), presented the lower tumor grade [G1: 83.3% (15/18) vs. 39.4% (13/33); G2: 11.1% (2/18) vs. 54.5% (18/33); G3: 5.6% (1/18) vs. 6.1% (2/33), Z=-2.766, P=0.006], were less likely to have serum gastrin which was 10 times higher than normal [11.8% (2/17) vs. 56.0% (14/33), χ(2)=8.396, P=0.004], had higher probability of complication with type 2 gastric neuroendocrine tumors (g-NET) [31.6% (6/19) vs. 3.0%(1/33), χ(2)=6.163, P=0.013], and had lower rate of liver metastasis [21.1% (4/19) vs. 51.5% (17/33), χ(2)=4.648, P=0.031). There was no obvious difference between sporadic gastrinomas and MEN1-related gastrinomas in endoscopic findings. Both types presented enlarged and swollen gastric mucosa under the stimulation of high gastric acid, and multiple ulcers in the stomach and duodenum could be seen. Gastrinoma with type 2 g-NET presented multiple polypoid raised lesions in the fundus and body of the stomach. (68)Ga-SSR-PET/CT scan had a 100% detection rate for both types while (18)F-FDG-PET/CT scan had a higher detection rate for sporadic gastrinoma compared with MEN1-related gastrinoma [57.9% (11/19) vs. 20.0% (3/15), χ(2)=4.970, P=0.026]. Among the patients with sporadic gastrinoma, 19 received surgical treatment, 1 underwent endoscopic submucosal dissection, 8 underwent transcatheter arterial embolization (TAE), and 5 underwent surgery combined with TAE. Among patients with MEN1-related gastrinoma, 13 received surgical treatment, and the other 6 received conservative treatment. The median follow-up of all the patients was 21.5 (1-129) months, and the 5-year survival rate was 88.4%. The 5-year survival rate of patients with sporadic and MEN1-related gastrinomas was 89.5% and 80.0% respectively (P=0.949). The 5-year survival rate of patients with and without liver metastasis was 76.2% vs. 100%, respectively (P=0.061). Conclusions: Compared with sporadic gastrinoma, MEN1-related gastrinoma has longer diagnosis delay, smaller tumor diameter, lower tumor grading, lower risk of liver metastasis, and is more likely to complicate with type 2 g-NET, while there is no difference in survival between the two tumor types.目的： 分析散发型与多发性内分泌腺瘤病1型（MEN1）相关型胃泌素瘤的临床特征，为临床诊治提供帮助。 方法： 采用回顾性队列研究方法。纳入有卓-艾综合征的临床表现且经病理确诊为神经内分泌肿瘤（NEN）的临床及随访资料完整的患者，排除仅有高胃酸分泌表现而无NEN证据者以及合并其他非NEN肿瘤者。根据上述标准，收集2003年4月至2020年12月期间，在中山大学附属第一医院就诊的52例胃泌素瘤患者的临床病理资料。符合胃泌素瘤诊断标准且满足以下条件之一的患者，被诊断为MEN1相关型胃泌素瘤：（1）经基因检测证实存在MEN1基因致病突变；（2）含有两个或两个以上内分泌腺体的NEN，即垂体、甲状旁腺、胸腺、胰腺以及肾上腺NEN；（3）发生NEN且至少一个一级亲属诊断为MEN1。其余胃泌素瘤定义为散发型胃泌素瘤。通过t检验和χ(2)检验进行统计学分析，比较散发型与MEN1相关型胃泌素瘤的临床病理特征、内镜下表现、影像学特征、治疗及预后情况。 结果： 52例胃泌素瘤患者中，散发型33例，MEN1相关型19例，散发型和MEN1相关型的主要临床表现均为腹泻（24/33，72.7%；17/19）和腹痛（19/33，57.6%；9/19）。与散发型胃泌素瘤相比，MEN1相关型诊断推迟时间更长[（7.4±4.9）年比（3.9±5.2）年，t=-2.355，P=0.022]；多发肿瘤比例更高[9/19比15.2%（5/33），χ(2)=6.361，P=0.012]；肿瘤最大径更短[（1.7±1.0）cm比（3.1±1.8）cm，t=2.942，P=0.005]；肿瘤分级更低[G1：15/18比39.4%（13/33）；G2：2/18比54.5%（18/33）；G3：1/18比6.1%（2/33），Z=-2.766，P=0.006]；血清胃泌素高于正常值10倍的比例更低[2/17比56.0%（14/25），χ(2)=8.396，P=0.004]；合并2型胃神经内分泌肿瘤（g-NET）的概率更高[6/19比3.0%（1/33），χ(2)=6.163，P=0.013）；出现肝转移比例更低[4/19比51.5%（17/33），χ(2)=4.648，P=0.031]。散发型与MEN1相关型的胃泌素瘤在内镜下无明显区别，均为高胃酸刺激下胃黏膜粗大、红肿，可见胃、十二指肠多发溃疡。合并2型g-NET的胃泌素瘤表现为胃底、胃体多发息肉样隆起性病变。(68)Ga-SSR-PET-CT扫描对两种类型的胃泌素瘤都有100%的检出率，但与MEN1相关型胃泌素瘤比较，(18)F-FDG-PET-CT扫描对散发型胃泌素瘤检出率更高[11/19比3/15，χ(2)=4.970，P=0.026]。在散发型胃泌素瘤患者中，有19例接受外科手术治疗，1例行内镜黏膜下剥离术，8例行肝动脉栓塞术（TAE），另有5例行手术联合TAE治疗。在MEN1相关型胃泌素瘤患者中，有13例接受外科手术治疗，另6例行保守治疗。全部患者中位随访21.5（1~129）个月，5年总体生存率为88.4%。散发型与MEN1相关型胃泌素瘤患者的5年总体生存率为89.5%和80.0%（P=0.949）；发生与未发生肝转移胃泌素瘤患者的5年总体生存率为76.2%比100%（P=0.061），差异均无统计学意义（均P>0.05）。 结论： 与散发型胃泌素瘤比较，MEN1相关型胃泌素瘤诊断延迟时间更长，肿瘤最大径更小，肿瘤分级更低，发生肝转移风险更低，且更易合并2型g-NET；但两者的生存相似。." @default.
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• W3206128979 title "[Comparison of clinical characteristics between sporadic gastrinoma and multiple endocrine neoplasia type 1-related gastrinoma]." @default.
• W3206128979 doi "https://doi.org/10.3760/cma.j.cn.441530-20210719-00291" @default.
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